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   Table of Contents      
CASE REPORT
Year : 2020  |  Volume : 68  |  Issue : 10  |  Page : 2305-2307

Multicentric recurrent uveal melanoma


1 Ophthalmic Plastic Surgery and Ocular Oncology Services, Centre for Sight, New Delhi, India
2 Vitreo-Retina Services, Centre for Sight, New Delhi, India
3 Ophthalmic Plastic Surgery, Ocular Oncology and Ocular Pathology Services, Centre for Sight, Hyderabad, Telangana, India

Date of Submission11-Mar-2020
Date of Acceptance06-May-2020
Date of Web Publication23-Sep-2020

Correspondence Address:
Dr. Vikas Menon
Centre for Sight, B-5/24, Safdarjung Enclave, New Delhi - 110 029
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/ijo.IJO_511_20

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  Abstract 


Uveal melanoma is a rare malignancy originating from melanocytes within the uveal tract of the eye. True multifocal uveal melanomas (>2melanomas in 1 eye) occurring in the same eye are very rare. We report a clinically and histologically well-documented case of a patient who presented with 3 uveal melanoma lesions in the same eye over a span of 2.5 years. The eye had to be enucleated finally and diagnosis confirmed histologically. This case report highlights the need for a close follow-up, even after successful treatment of the presenting lesion in uveal melanomas.

Keywords: Brachytherapy, choroidal melanoma, ciliarybody melanoma, enucleation


How to cite this article:
Menon V, Thulasidas M, Narula R, Mulay K, Honavar SG. Multicentric recurrent uveal melanoma. Indian J Ophthalmol 2020;68:2305-7

How to cite this URL:
Menon V, Thulasidas M, Narula R, Mulay K, Honavar SG. Multicentric recurrent uveal melanoma. Indian J Ophthalmol [serial online] 2020 [cited 2020 Oct 20];68:2305-7. Available from: https://www.ijo.in/text.asp?2020/68/10/2305/295744



Uveal melanoma is a rare malignancy originating from melanocytes within the uveal tract of the eye.[1] Choroidal melanoma is the most common subtype of uveal melanoma. Ciliary body melanoma is found rarely, reported in 1 of 10 cases of all intraocular melanomas.[2] Unilateral double melanomas have been reported in association with ocular melanocytosis and as a syndrome with other systemic malignant neoplasms.[3],[4] True multifocal uveal melanomas (>2 melanomas in 1 eye as opposed to double) are very rare, with only two cases reported to date.[5],[6] We report a patient with three uveal melanoma lesions in the same eye.


  Case Report Top


A 59-year-old man presented to us 4 years ago with flashes in left eye (LE). The best corrected visual acuity (BCVA) was 6/6 in both eyes. Anterior segment examination and intraocular pressure (IOP) were within normal limits. Fundus examination of LE revealed a dome shaped pigmented mass with surface exudates and hemorrhage in the supero-nasal quadrant [Figure 1]a. B-scan ultrasonography, fluorescein angiography, and magnetic resonance imaging (MRI) orbit findings were consistent with choroidal melanoma. B-scan ultrasonography showed a choroidal mass of 11 × 6.1 mm with acoustic hollowing. He had history of hypertension for last 5 years for which he was under treatment. Family history was negative for malignancy. He underwent plaque brachy therapy (Ruthenium-106), 10,000 cGy to the tumor apex at 6mm, following which the lesion regressed [Figure 1]b. He developed mild radiation retinopathy in the treated eye and was on regular 3-monthly follow-up.
Figure 1: (a): Choroidal melanoma: a pigmented dome shaped choroidal mass with surface exudates and hemorrhage in the supero-nasal quadrant of left eye. (b): Regressed melanoma following plaque brachy therapy. (c): Ciliary body melanoma in the periphery of nasal quadrant of left eye. (d): Ciliary body melanoma in the periphery of temporal quadrant of left eye

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Patient was doing well for 2.5 years, asymptomatic without any evidence of local or systemic recurrence. BCVA was 6/6 in right eye (RE) and 6/12 in LE. During a routine follow-up, fundus examination of LE showed new dome shaped pigmented masses in the periphery of temporal and nasal quadrants. The supero-nasal primary tumor appeared all scarred and regressed [Figure 1]c and [Figure 1]d. A suspicion of recurrent uveal melanoma was made. Ultrasound biomicroscopy revealed solid masses in nasal (8 × 8 × 5.37 mm) and temporal (11 × 8 × 3.8 mm) ciliary body areas with low internal reflectivity [Figure 2]a and [Figure 2]b. MRI orbits showed the presence of mass lesions along the temporal and nasal aspects of the left globe, hyper-intense on T1 and hypo-intense on T2 weighted images. Positron emission tomography scan, ultrasound abdomen, chest x-ray, and liver function tests were done. Systemic screening was negative for metastases elsewhere in the body. A diagnosis of recurrent multi-centric uveal melanoma was made. Enucleation of LE with orbital implant was done. Histopathological and immunohistochemical analysis confirmed multi-centric malignant melanoma, epithelioid type [Figure 2]c and [Figure 2]d involving ciliary body and choroid, positive for HMB-45. The intervening areas of choroid were normal on serial sections which ruled out underlying diffuse melanoma. Genetic analysis showed chromosome 6q deletion. The patient is on 3-monthly follow-up without any metastasis at the end of 18 months.
Figure 2: (a): Nasal ciliary body melanoma (8 × 8 × 5.37mm) with low internal reflectivity on ultrasound biomicroscopy.(b): Temporal ciliary body melanoma (11 × 8 × 3.8 mm) with low internal reflectivity on ultrasound biomicroscopy.(c): Haematoxylin and eosin (H and E) stained section of a mushroom shaped, endophytic ciliary body melanoma, under low magnification (4×).(d): H and E stained section of melanoma showing epithelioid cells with abundant eosinophilic cytoplasm, large eccentrically placed vesicular nucleus and prominent nucleolus, under high magnification (×400)

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  Discussion Top


A case in which a patient's eye is harboring more than two uveal melanomas is indeed a rare occurrence. To our knowledge, true multifocal cases are reported only twice with one lacking histological evidence and the other lacking a cytogenetic proof.[5],[6]

Rosen and Moulton (1953) reported a case of one large and four small flat discrete tumor nodules seen in the vicinity of the optic nerve head. They concluded that at least two of the lesions are completely independent and the exact number of lesions cannot be stated in the absence of serial sections through the whole eye.[5]

Bluementhal and Pe'er (1999) published a case of three separate uveal melanomas in a single eye but were unaware of a genetic or other marker that could unequivocally ascertain whether the distinct tumors originated from a single cell line.[6]

We report on a histologically verified case of a single eye harboring three non-contiguous uveal melanomas of epithelioid type positive for HMB-45 with genetic analysis showing chromosome 6q deletion. The presence of several uveal neoplasms may be secondary to metastatic dissemination from a single primary tumor, but its possibility would be less likely in the case described, as there was no other positive systemic focus of metastasis at the end of 18 months follow-up.

Recent studies have shown that Chromosome 3 deletion, BAP1 loss, chromosome 8q gain, chromosome 1p loss, and chromosome 6q loss are associated with poor prognosis. Disomy 3 and chromosome 6p gain are associated with a better prognosis.[7] In fact, it is now recognized that genetic changes play a very significant role in the growth, as well as metastatic potential of these tumors.[8],[9]

The prognosis of uveal melanoma can be predicted by clinical, histopathological, and cytogenetic markers.[10] This case of multi-centric recurrent uveal melanoma of epithelioid type involving ciliary body and choroid with chromosome 6q loss indicates poor prognosis. The most effective way to improve the prognosis is early detection of melanoma and close follow-up with systemic monitoring.


  Conclusion Top


Development of 3 uveal melanomas in a single eye is very rare. To the best of our knowledge, this is the first reported case of true multifocal uveal melanomas with histopathological and cytogenetic evidence. Such patients with unfavorable genetic mutations have high risk of metastasis and hence closer follow-up is suggested.

Acknowledgements

Dr. Vikas Khetan, Sankara Nethralaya, Chennai.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.



 
  References Top

1.
Spagnolo F, Caltabiano G, Queirolo P. Uveal melanoma. Cancer Treat Rev 2012;38:549-53.  Back to cited text no. 1
    
2.
Oittinen HA-L, O'Shaughnessy M, Cullinane AB, Keohane C. Malignant melanoma of the ciliary body presenting as extraocular metastasis in the temporalis muscle. J ClinPathol 2007;60:834-5.  Back to cited text no. 2
    
3.
Rao R, Honavar SG, Mulay K. Multifocal choroidal melanoma in oculodermalmelanocytosis in an Asian Indian. Indian J Ophthalmol 2019;67:2089–91.  Back to cited text no. 3
    
4.
Barr CC, Zimmerman LE, Curtin VT, Font RL. Bilateral diffuse melanocytic uveal tumors associated with systemic malignant neoplasms: A recently recognized syndrome. Arch Ophthalmol 1982;100:249-55.  Back to cited text no. 4
    
5.
Rosen DA, Moulton GN. Multiple malignant melanomas in one eye. Am J Ophthalmol 1953;36:73-5.  Back to cited text no. 5
    
6.
Blumenthal EZ, Pe'er J. Multifocal choroidal malignant melanoma: At least 3 melanomas in one eye. Arch Ophthalmol 1999;117:255-8.  Back to cited text no. 6
    
7.
Onken MD, Worley LA, Tuscan MD, Harbour JW. An accurate, clinically feasible multi-gene expression assay for predicting metastasis in uveal melanoma. J MolDiagn 2010;12:461-8.  Back to cited text no. 7
    
8.
Augsburger JJ, Correa ZM, Trichopoulos N. An alternative hypothesisfor observed mortality rates due to metastasis after treatment of choroidal melanomas of different sizes. Trans Am OphthalmolSoc 2007;105:54-9.  Back to cited text no. 8
    
9.
Damato B, Coupland SE. A reappraisal of the significance of largest basal diameter of posterior uveal melanoma. Eye 2009;23:2152-60.  Back to cited text no. 9
    
10.
Kaliki S, Shields CL, Shields JA. Uveal melanoma: Estimating prognosis. Indian J Ophthalmol 2015;63:93-102.  Back to cited text no. 10
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